Overview
Definition
A non-atherosclerotic, inflammatory vaso-occlusive disorder of small and medium-sized arteries and veins in the upper and lower extremities.
Epidemiology
Most common in males under 45 who are heavy tobacco smokers.
Etiology & Risk Factors
- Hypersensitivity to tobacco antigens
- Inflammatory thrombosis of vascular lumen
Clinical Symptoms
- Claudication in instep or arch of foot
- Raynaud's phenomenon
- Superficial migratory thrombophlebitis
- Rest pain
- Digital gangrene
Clinical Approach
Diagnosis
- Angiography showing segmental occlusions ('corkscrew collaterals')
- Biopsy (rarely done but diagnostic)
- Normal lab work (no autoimmune markers)
Management
- ABSOLUTE and immediate smoking cessation (nicotine replacements can perpetuate disease)
- Iloprost (prostacyclin analogue)
- Surgical revascularization is rarely feasible due to distal nature
Complications
- Amputation of fingers/toes
- Digital gangrene
- Refractory ulcers